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POEMS Syndrome.

Arnaud Jaccard1, Virginie Pascal2, Laurent Magy3

  • 1Department of Hematology and Cellular Therapy, National Reference Center "AL Amyloidosis and Other Monoclonal Immunoglobulin Deposit Diseases, University Hospital of Limoges, Limoges, France; CNRS UMR-7276, INSERM U1262, Team 3 BioPIC of CRIBL, University of Limoges, Limoges, France.

Presse Medicale (Paris, France : 1983)
|January 23, 2025
PubMed
Summary
This summary is machine-generated.

POEMS syndrome, a rare disorder involving B-cell proliferation, presents with diverse symptoms. Early diagnosis and treatment, including radiation or systemic therapies like ASCT, lenalidomide, and bortezomib, are vital for managing this condition.

Keywords:
Autologous stem cell transplantationCastleman's diseaseMGCSPOEMS syndromeRadiationTreatmentVEGF

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Area of Science:

  • Hematology
  • Neurology
  • Oncology

Background:

  • POEMS syndrome is a rare multisystem disorder characterized by monoclonal B-cell proliferation.
  • Clinical manifestations are diverse, including neuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes, alongside potential bone lesions and organ impairments.

Purpose of the Study:

  • To summarize the key features, diagnostic challenges, and treatment strategies for POEMS syndrome.
  • To highlight the importance of early intervention for optimal patient outcomes.

Main Methods:

  • Review of existing literature on POEMS syndrome.
  • Analysis of diagnostic criteria and treatment modalities.

Main Results:

  • Diagnosis is often delayed due to the syndrome's rarity and varied presentation.
  • Treatment varies based on disease extent, ranging from radiation to systemic therapies like autologous stem cell transplantation (ASCT), lenalidomide, and bortezomib.
  • Lenalidomide and bortezomib show rapid efficacy in treating neuropathy.

Conclusions:

  • Prompt diagnosis and treatment initiation are crucial for managing POEMS syndrome.
  • Multidisciplinary care, including physiotherapy, is essential for neurological recovery.
  • Emerging therapies offer promising outcomes for patients with POEMS syndrome.